Practice parameter for repetitive nerve stimulation and single fiber EMG evaluation of adults with suspected myasthenia gravis or Lambert–Eaton myasthenic syndrome: Summary statement

Chiou‐Tan, Faye Y.; Tim, Richard W.; Gilchrist, James M.; Weber, Cheryl F.; Wilson, John R.; Benstead, Timothy; Braker, Arlene M.; Caress, James B.; Chokroverty, Sudhansu; Hackett, Earl R.; Harmon, Robert L.; Hughes, Bernadette A.; Kothari, Milind J.; Lachman, Tim; Malamut, Richard; Marciniak, Christina; Miller, Robert G.; Nelson, Kevin R.; Olney, Richard K.; Patel, Atul T.; Quartly, Caroline; Ryan, Karen S.

doi:10.1002/mus.1139

Cited by 112 publications

(39 citation statements)

References 29 publications

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“…All patients gave informed consent to inclusion in the study, which was approved by the local Ethics Committee. The diagnosis of MG was made on the typical history and signs of fluctuating weakness of voluntary muscles, a decremental pattern on repetitive nerve stimulation, and/or an increase in jitter on single-muscle fiber studies [10]. Age at disease onset, presence of serum anti-AChR or anti-muscle-specific kinase (MuSK) antibodies, and presence of thymoma, as assessed by chest CT scan, were additional criteria used for identifying MG subtypes.…”

Section: Methodsmentioning

confidence: 99%

Association of HLA-DQB105:02 and DRB116 Alleles with Late-Onset, Nonthymomatous, AChR-Ab-Positive Myasthenia Gravis

Testi

Terracciano

Guagnano

et al. 2012

Autoimmune Diseases

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An association of several HLA alleles with myasthenia gravis (MG) has been reported. Aim of this work was to analyze the HLA allele profile in a survey of 76 unselected Italian MG patients and in a subgroup characterized by disease onset after the age of 50 years, absence of thymoma, and presence of antiacetylcholine receptor antibodies. We defined this subgroup by the acronym LOAb. Typing was performed at low resolution for HLA-A, -B, and -DRB1 loci with sequence-specific oligonucleotide probe (PCR-SSO); at high resolution for HLA-DQB1 locus by PCR with sequence-specific primers (PCR-SSPS). HLA allele frequencies were compared with 100 healthy controls. No correlation was observed between MG and the studied HLA class I alleles. On the contrary, a strong positive association was found for the HLA class II alleles DQB1∗05:02 (P c = 0.00768) and DRB1∗16 (P c = 0.0211) in the LOAb subgroup (n = 27) of MG patients. Association between DQB1∗05:02 and some subtypes of MG has been previously reported but not in patients with the LOAb characteristics. Therefore, the HLA allele DQB1∗05:02 might be considered as a susceptibility marker for LOAb among Italians.

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Section: Methodsmentioning

confidence: 99%

Association of HLA-DQB105:02 and DRB116 Alleles with Late-Onset, Nonthymomatous, AChR-Ab-Positive Myasthenia Gravis

Testi

Terracciano

Guagnano

et al. 2012

Autoimmune Diseases

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“…All patients had RNS in the O.O., nasalis and ADQ, and the recommendations of AAEM for RNS [15] were followed: (1) anticholinesterase medication was withheld 12 h prior to testing, (2) the nerves were stimulated at an intensity of 30% above the intensity required for maximal compound muscle action potential amplitude for single stimuli, (3) 1–2 trains of 5 stimuli at 3 Hz were given at rest followed by 40 s of maximal voluntary contraction, (4) trains of 5 stimuli at 3 Hz were given immediately after the exercise and at regular intervals of 1 min for the following 5 min, and (5) skin temperature was maintained above 32°C. The amplitude of the fourth or fifth intravolley waveform was measured peak to peak.…”

Section: Methodsmentioning

confidence: 99%

Repetitive Nerve Stimulation of Facial and Hypothenar Muscles: Relative Sensitivity in Different Myasthenia Gravis Subgroups

Kokotis¹,

Karandreas²

2011

Eur Neurol

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Aim: To assess the utility of repetitive nerve stimulation (RNS) in facial and hypothenar muscles in the clinical groups of myasthenia gravis (MG). Patients and Methods: We performed RNS study in the orbicularis oculi (O.O.), nasalis and abductor digiti quinti (ADQ) in 115 consecutive myasthenic patients and classified them according to the classifications of the Myasthenia Gravis Foundation of America. Patients were classified into three groups: group 1, group 2 (IIa, IIIa and IVa) and group 3 (IIb, IIIb and IVb). Results: RNS was abnormal in 95 patients (82.6%): 78.3% in the O.O., 66.1% in the nasalis and 19.1% in the ADQ. Both facial muscles were statistically more sensitive than the ADQ in all groups of patients. RNS in the O.O. was more frequently abnormal than in the nasalis only in group 1. Sensitivity to acetylcholine antibodies in myasthenic patients was 84%. Acetylcholine receptor (AChR) and muscle-specific tyrosine kinase antibodies were present in 96.7% of the patients with abnormal RNS in both facial muscles. Single-fiber electromyogram (SFEMG) was abnormal in 91.3% of the tested patients. One of the three tests used for the diagnosis of MG (AChR antibodies, SFEMG, RNS) was abnormal in 99.1% of the patients. Discussion: O.O. is the most sensitive muscle in all groups of MG followed by nasalis, while the ADQ is the muscle with the lowest sensitivity. Facial muscles, especially the O.O., should be the first to be tested in MG. The negativity of all tests (RNS, AChR antibodies, SFEMG) should question the diagnosis of MG, even in the presence of symptoms consistent with MG.

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“…The incremental response was not quantified in the article. Further recommendations added that increments greater than 100% should be observed as more accurate (gold standard) to confirm the diagnosis of LEMS 10 . Even after 60 years, the main electrophysiological criteria for LEMS described in Eaton and Lambert's pioneering article are still useful in the LEMS diagnosis.…”

Section: Eaton and Lambert's Pioneering Articlementioning

confidence: 99%

Lambert-Eaton myasthenic syndrome: the 60th anniversary of Eaton and Lambert's pioneering article

Lorenzoni

Kay

Werneck

et al. 2018

Arq. Neuro-Psiquiatr.

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This historical review describes the contribution of Drs. Lee M. Eaton and Edward H. Lambert to the diagnosis of myasthenic syndrome on the 60th anniversary of their pioneering article (JAMA 1957) on the disease. There are important landmarks in their article on a disorder of the neuromuscular junction associated with thoracic neoplasm and the electrophysiological criteria for Lambert-Eaton myasthenic syndrome (LEMS). After 60 years, the main electrophysiological criteria described in Eaton and Lambert's pioneering article are still currently useful in the diagnosis of LEMS.

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Practice parameter for repetitive nerve stimulation and single fiber EMG evaluation of adults with suspected myasthenia gravis or Lambert–Eaton myasthenic syndrome: Summary statement

Cited by 112 publications

References 29 publications

Association of HLA-DQB105:02 and DRB116 Alleles with Late-Onset, Nonthymomatous, AChR-Ab-Positive Myasthenia Gravis

Association of HLA-DQB105:02 and DRB116 Alleles with Late-Onset, Nonthymomatous, AChR-Ab-Positive Myasthenia Gravis

Repetitive Nerve Stimulation of Facial and Hypothenar Muscles: Relative Sensitivity in Different Myasthenia Gravis Subgroups

Lambert-Eaton myasthenic syndrome: the 60th anniversary of Eaton and Lambert's pioneering article

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Practice parameter for repetitive nerve stimulation and single fiber EMG evaluation of adults with suspected myasthenia gravis or Lambert–Eaton myasthenic syndrome: Summary statement

Cited by 112 publications

References 29 publications

Association of HLA-DQB1*05:02 and DRB1*16 Alleles with Late-Onset, Nonthymomatous, AChR-Ab-Positive Myasthenia Gravis

Association of HLA-DQB1*05:02 and DRB1*16 Alleles with Late-Onset, Nonthymomatous, AChR-Ab-Positive Myasthenia Gravis

Repetitive Nerve Stimulation of Facial and Hypothenar Muscles: Relative Sensitivity in Different Myasthenia Gravis Subgroups

Lambert-Eaton myasthenic syndrome: the 60th anniversary of Eaton and Lambert's pioneering article

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Association of HLA-DQB105:02 and DRB116 Alleles with Late-Onset, Nonthymomatous, AChR-Ab-Positive Myasthenia Gravis

Association of HLA-DQB105:02 and DRB116 Alleles with Late-Onset, Nonthymomatous, AChR-Ab-Positive Myasthenia Gravis